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Hamaoka et al. Journal of Medical Case Reports (2016) 10:271 DOI 10.1186/s13256-016-1055-8

CASE REPORT Open Access Intestinal in a patient with hypertrophic obstructive cardiomyopathy: a case report Takuto Hamaoka1*, Wataru Omi2, Yoshiteru Sekiguti2, Shigeo Takata2, Shuichi Kaneko1, Oto Inoue2, Shinichiro Takashima2, Hisayoshi Murai1, Soichiro Usui1, Takeshi Kato1, Hiroshi Furusho1 and Masayuki Takamura1

Abstract Background: Intestinal angina is characterized by recurrent postprandial abdominal pain and anorexia. Commonly, these symptoms are caused by severe stenosis of at least two vessels among the celiac and mesenteric arteries. However, intestinal perfusion is affected not only by the degree of arterial stenosis but also by systemic perfusion. We experienced a unique case of intestinal angina caused by relatively mild stenosis of the abdominal arteries complicated with hypertrophic obstructive cardiomyopathy. Case presentation: We report an 86-year old Japanese man with hypertrophic obstructive cardiomyopathy and advanced who was diagnosed with intestinal angina. Computed tomography showed mild stenosis of the celiac artery and severe stenosis of the inferior mesenteric artery, and these lesions were relatively mild compared with other reports. A dual-chamber pacemaker with right ventricular apical pacing was implanted to improve the obstruction of the left ventricular outflow tract. After implantation, the patient’sabdominalsymptoms diminished markedly, and improvement of the left ventricular outflow tract obstruction was observed. Conclusions: Although intestinal angina is generally defined by severe stenosis of at least two vessels among the celiac and mesenteric arteries, the present case suggests that hemodynamic changes can greatly affect intestinal perfusion and induce intestinal angina in the presence of mild stenosis of the celiac and mesenteric arteries. Keywords: Intestinal angina, Hypertrophic obstructive cardiomyopathy, Advanced atrioventricular block, Case report

Background tract (LVOT), resulting in an increased LVOT pressure Intestinal angina is caused by chronic intestinal gradient. On the other hand, increases the characterized by recurrent postprandial abdominal pain cardiac afterload, which inhibits the collapse of LVOT and and a decrease in body weight with anorexia [1]. The results in a decreased LVOT pressure gradient [3]. symptom is commonly manifested when at least two ves- In this report, we describe a unique case of intestinal sels of the celiac and mesenteric arteries have developed angina complicated with HOCM and abruptly worsened moderate to severe stenosis or occlusion. Hypertrophic by advanced atrioventricular (AV) block in the presence obstructive cardiomyopathy (HOCM) is associated with a of mild stenosis of the abdominal arteries. reduction in and systemic perfusion fluctu- ated by multiple hemodynamic conditions [2] such as hypovolemia or high blood pressure. Hypovolemic condi- Case presentation tions lead to a reduction in left ventricular volume and An 86-year old Japanese man was admitted to our hospital the narrowing or collapse of the left ventricular outflow because of worsening postprandial abdominal pain, an- orexia, and general malaise. He was previously diagnosed * Correspondence: [email protected] with HOCM on cardiac magnetic resonance imaging and 1 Department of Disease Control and Homeostasis, Graduate School of , but because he was asymptomatic, he Medical Science, Kanazawa University, 13-1 Takara-machi, Kanazawa 920-8641, Japan was not taking oral medications. He had been suffering Full list of author information is available at the end of the article from repetitive and paroxysmal upper abdominal pain for

© 2016 The Author(s). Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Hamaoka et al. Journal of Medical Case Reports (2016) 10:271 Page 2 of 5

1 year. His symptoms occurred approximately 15 minutes An electrocardiogram revealed advanced AV block and after eating and usually disappeared within 1 hour and left high voltage with ST-T change; AV disturbance had were sometimes accompanied by watery diarrhea. Once not been detected previously (Fig. 3). Transthoracic echo- his abdominal attacks occurred, he had great difficulties cardiography showed significant obstruction of the LVOT with oral intake due to postprandial pain, but his symp- (pressure gradient, 35 mmHg) and mitral regurgitation toms disappeared upon fluid replacement for several days. with systolic anterior motion of the anterior mitral leaflet He presented to our department because of worsening (Fig. 4). These echocardiographic findings were similar to and more sustained symptoms within the week prior. those of echocardiography performed the previous year He was emaciated and exhausted and had lost 7 kg in on our patient. the last 1 year. A physical examination showed a low CT angiography was classified as mild to account for blood pressure (90/60 mmHg) and (40 beats our patient’s severe postprandial pain, since our patient’s per minute). He had no signs of disorientation or paraly- superior mesenteric artery, which had the widest sis. A holosystolic ejection murmur at the fourth left intestinal perfusion area, was patent in this patient; how- sternal border was auscultated, and abdominal palpita- ever, in many previous reports concerning intestinal an- tion showed no abnormal findings. The murmur was gina, stenosis of this artery was demonstrated, and accentuated by the Valsalva maneuver and walking. La- angioplasty was performed [4]. Thus, it was suspected boratory data showed slight anemia (hemoglobin 11.5 g/ that hemodynamic failure contributed to the pathophysi- day), hypoalbuminemia (3.8 g/dL, normal range 3.8– ology. That is, under the chronic low output condition 5.3 g/dL), and mild renal dysfunction (serum creatinine of uncontrolled HOCM, factors such as occasional dehy- (Cre) 1.33 mg/dL, normal range 0.6–1.2 mg/dL; blood dration and bradycardia had reduced systemic perfusion, urea nitrogen (BUN) 39 mg/dL, normal range 9–23 mg/ especially that of intestinal blood flow. We performed a dL; estimated glomerular filtration rate (eGFR) 39.5 mL/ pacemaker implantation with right ventricular apical minute/1.73 m2), and no signs of dyslipidemia or dia- pacing to improve our patient’s hemodynamic condition. betes mellitus (low density lipoprotein cholesterol After dual-chamber pacemaker implantation, pacing AV 98 mg/dL; glycosylated hemoglobin 5.3 %; fasting blood delay was optimized for a minimum LVOT pressure gra- glucose 100 mg/dL). Our patient’s activated partial dient and regurgitation under echocardio- thromboplastin time (33.1 seconds) and prothrombin graphic estimations (AV delay 100 milliseconds for time (13.0 seconds) were within the normal ranges. Gas- LVOT 14.6 mmHg, Fig. 4). Following these procedures, troscopy and colonoscopy examinations detected no ab- our patient’s appetite and daily activity improved dra- normal findings, while a computed tomography (CT) matically. In addition, his blood pressure improved sig- scan with contrast enhancement demonstrated moderate nificantly, his renal function became normal (Cre, stenosis of the celiac artery and severe stenosis of the in- 0.83 mg/dL; BUN, 25 mg/dL; eGFR, 66.2 mL/minute/ ferior mesenteric artery (Fig. 1). In addition, significant 1.73 m2), and a chest X-ray examination showed signifi- dilatation of the bowels was observed by CT and chest cant improvement of intestinal dilatation (Fig. 2), sug- X-ray examinations (Fig. 2). gesting increase in systemic and intestinal perfusion.

Fig. 1 Enhanced computed tomography showed severe atherosclerosis of the arteries. The aorta showed broad calcifications, and the celiac artery showed moderate stenosis, although the lumen of the SMA was relatively patent. In addition, stenosis of the IMA was very severe. IMA inferior mesenteric artery, SMA superior mesenteric artery Hamaoka et al. Journal of Medical Case Reports (2016) 10:271 Page 3 of 5

artery and disturb blood flow (median arcuate ligament syndrome) [5, 6]. Intestinal circulation consists of an abundant collateral blood supply, and chronic intestinal ischemia is associated with high-grade stenosis or occlusion of two or more of the three major vessels: the celiac artery, the superior and inferior mesenteric arter- ies [7, 8]. In our case, the arterial lesions were relatively mild compared with previous reports [9, 10]; thus, we hypothesized that our patient’s major symptoms were not due to his arterial lesions alone. Specifically, our patient’s symptoms depended greatly on his hemodynamic condition, which caused a flow dis- crepancy between demand and supply [1]. Once an ab- Fig. 2 Chest X-ray examinations pre-PMI demonstrated significant dominal attack occurred, our patient’s blunted oral dilatation of the bowels; however, this dilatation was improved after PMI with right ventricular apical pacing. PMI pacemaker implantation intake exacerbated repetitive postprandial attacks; only fluid replacement led to remission. Furthermore, ad- vanced AV block abruptly worsened his condition and Finally, he has been completely free from abdominal pacemaker implantation with right ventricular apical symptoms in the last 3 months. pacing resulted in complete remission of his symptoms. In patients with HOCM, dehydration augments LVOT Discussion obstruction and mitral regurgitation, leading to reduced Classically, chronic intestinal angina is caused by a re- cardiac output [2]. Pacemaker implantation with right duction in mesenteric blood flow [1], and the patho- ventricular apical pacing increases cardiac output by op- physiology of most cases is atherosclerotic stenosis of timizing chronotropic action as well as by lowering the the celiac and mesenteric arteries. Arterial dissection, LVOT gradient in HOCM patients [11, 12]. Nonocclu- fibromuscular dysplasia, and vasculitis are included as sive mesenteric ischemia is a type of intestinal ischemia rare etiologies of arterial narrowing, and the median ar- attributed to hemodynamic failure, sometimes without cuate ligament of the diaphragm can compress the celiac arterial stenosis or occlusion. Vasospasm of mesenteric

Fig. 3 An electrocardiogram pre-PMI showed severe bradycardia with advanced AV block (approximately 40 beats/minute). After PMI, bradycardia was improved. AV atrioventricular, PMI pacemaker implantation Hamaoka et al. Journal of Medical Case Reports (2016) 10:271 Page 4 of 5

Fig. 4 Transthoracic echocardiography demonstrated severe MR with SAM and LVOT obstruction (LVOT pressure gradient, 35 mmHg) pre-PMI. After PMI, transthoracic echocardiography showed significant improvements in MR and LVOT obstruction (LVOT pressure gradient, 14.6 mmHg). LVOT left ventricular outflow tract, MR mitral valve regurgitation, SAM systolic anterior motion vessels is assumed to be a major pathophysiological and myectomy in improving the long-term prognosis of acute homeostatic response used to maintain systemic HOCM patients was reported previously [14], this treat- circulation at the expense of the splanchnic blood supply ment is well known for its high mortality rate, with older in critically ill conditions, such as cardiogenic age being a risk factor [15]. In this case, pacemaker im- [13]. In our case, the patient complained mainly of long- plantation was selected as the treatment, because our standing postprandial abdominal pain without any signs patient’s systemic circulation and abdominal symptoms of severe circulatory failure in other organs. He was later were clearly exacerbated by bradycardia from AV block. diagnosed with uncontrolled HOCM complicated with Some limitations were observed in this case report. No stenosis of the celiac and mesenteric arteries, and his intestinal hemodynamic parameters were evaluated, be- symptoms reflected excessive hemodynamic fluctuation cause abdominal ultrasound was unable to detect the specific to HOCM. Generally, easily digestible meals or celiac and mesenteric arterial flows. Furthermore, our vasodilators are used as supportive therapy, while the patient was very emaciated and unable to undergo cath- definitive treatment for intestinal angina is revasculariza- eter examination. However, after pacemaker implant- tion by surgery or catheterization [13]. However, our ation, his symptoms, intestinal dilatation, and renal patient was too old and emaciated to undergo these in- function improved without fluid replacement or add- vasive treatments. Although he underwent pacemaker itional medications, which reflected improvement of his implantation, it was less invasive than was revasculariza- systemic circulation. tion therapy. HOCM is commonly treated with oral medications, such as calcium channel blockers, β- adrenoreceptor blockers, and antiarrhythmic agents in- Conclusions cluded in group Ia of the Vaughan-Williams criteria, to This case suggests that hemodynamic changes could reduce myocardial oxygen consumption and inhibit left greatly affect intestinal perfusion in the presence of ventricular hypercontraction. Percutaneous transluminal mild stenosis of the celiac and mesenteric arteries. septal myocardial , septal myectomy, and pace- Thus, it should be recognized that intestinal angina maker implantation are other potential treatments if oral might be a symptom of , espe- therapy is not successful. Although the efficacy of septal cially cardiac failure. Hamaoka et al. Journal of Medical Case Reports (2016) 10:271 Page 5 of 5

Abbreviations 14. Robbins RC, Stinson EB. Long-term results of left ventricular myotomy and AV: atrioventricular; BUN: blood urea nitrogen; Cre: creatinine; CT: computed myoectomy for obstructive hypertrophic cardiomyopathy. J Thorac tomography; eGFR: estimated glomerular filtration rate; HOCM: hypertrophic Cardiovasc Surg. 1996;111:586–94. obstructive cardiomyopathy; LVOT: left ventricular outflow tract 15. Smedira NG, Lytle BW, et al. Current effectiveness and risks of isolated septal myectomy for hypertrophic obstructive cardiomyopathy. Ann Thorac Surg. – Acknowledgements 2008;85:127 33. We gratefully acknowledge the patient described in this report.

Funding The authors declare no funding associated with this report.

Authors’ contributions TH compiled the case and drafted the initial manuscript. OI, ST, and TK contributed significantly to writing the manuscript. WO, YS, and ST performed the initial literature review, clinical diagnosis, and treatment of the case. SU, HF, HM, SK, and MT proofread the manuscript prior to submission. All authors read and approved the final manuscript.

Competing interests The authors declare that they have no competing interests.

Consent for publication Written informed consent for publication of this case report and any accompanying images was obtained from the patient. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Ethics approval and consent to participate Not applicable since this case report was retrospective and observational.

Author details 1Department of Disease Control and Homeostasis, Graduate School of Medical Science, Kanazawa University, 13-1 Takara-machi, Kanazawa 920-8641, Japan. 2Department of , Kanazawa Municipal Hospital, Kanazawa, Japan.

Received: 17 June 2016 Accepted: 5 September 2016

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